Objective: Kawasaki disease (KD) is an intense life-threatening vasculitis. The diagnosis of Kawasaki disease is made by clinical criteria. This disease is a common rheumatologic disease in Iran. The aim of this study was describe the demographics and clinical features of KD in Iranian patients. Material & Methods: A review was conducted for all cases of KD treated at Pediatric rheumatology department in Children's Medical Center between January 1994 and July 2004. The diagnostic criteria for typical Kawasaki were based on the criteria of the Japan Kawasaki Disease Research Committee. Atypical or incomplete KD has been described in which patients not strictly meeting the diagnostic criteria but have coronary artery changes. Color doppler echocardiograms were done at the time of diagnosis, 14 to 21 days, 60 days, and 1 year after treatment. Findings: One hundred fifty nine patients were identified. One hundred twenty five children (78.6%) fulfilled criteria for typical KD. Echocardiographic abnormalities were found in 30 cases (18.9%), including 9.6% with typical and 46.1% with atypical Kawasaki. The incidence of atypical Kawasaki in our study was about 22%. Coronary arteries aneurysms found in 66.7% and 33.3% was other abnormalities. Male to female ratio was more in patients with cardiac complication (2.3:1).Conclusion: Kawasaki disease should be considered in any infants or child (especially less than 2 years old) with a prolonged febrile illness. Demographic features of our patients were similar to reports from other country. The incidence of atypical Kawasaki in our study was about 22%.